Neurology Flashcards

Question

The typical history of a subarachnoid haemorrhage is a sudden onset occipital headache that occurs during strenuous activity such as weight lifting or sex. This occurs so suddenly that it is known as a what + name 3 other features ?

Answer 1

A thunderclap headache. It is is described like being hit really hard on the back of the head. Other features are: - Neck stiffness - Photophobia - Neurological symptoms such as visual changes, speech changes, weakness, seizures and loss of consciousness

Answer 2

- Hypertension - Smoking - Excessive alcohol consumption - Cocaine use - Family history

Answer 3

- Black patients - Female patients - Age 45-70

Answer 4

- Cocaine use - Sickle cell anaemia - Connective tissue disorders (such as Marfan syndrome or Ehlers-Danlos) - Neurofibromatosis - Autosomal dominant polycystic kidney disease

Answer 5

CT head is the first line investigation. Immediate CT head is required. Blood will cause hyperattenuation in the subarachnoid space. Lumbar puncture is used to collect a sample of the cerebrospinal fluid if the CT head is negative. CSF can be tested for signs of subarachnoid haemorrhage: - Red cell count will be raised. If the cell count is decreasing in number over the samples, this could be due to a traumatic lumbar puncture. - Xanthochromia (the yellow colour of CSF caused by bilirubin) Angiography (CT or MRI) can be used once a subarachnoid haemorrhage is confirmed to locate the source of the bleeding.

Answer 6

- Patients should be managed by a specialist neurosurgical unit. Patients with reduced consciousness may require intubation and ventilation. Supportive care as part of a multi-disciplinary team is important with good nursing, nutrition, physiotherapy and occupational therapy involved during the initial stages and recovery. - Surgical intervention may be used to treat aneurysms. The aim is to repair the vessel and prevent re-bleeding. This can done by coiling, which involves inserting a catheter into the arterial system (taking an “endovascular approach”), placing platinum coils into the aneurysm and sealing it off from the artery. An alternative is clipping, which involves cranial surgery and putting a clip on the aneurysm to seal it. - Nimodipine is a calcium channel blocker that is used to prevent vasospasm. Vasospasm is a common complication that can result in brain ischaemia following a subarachnoid haemorrhage. - Lumbar puncture or insertion of a shunt may be required to treat hydrocephalus. - Antiepileptic medications can be used to treat seizures.

Answer 7

Multiple sclerosis (MS) is a chronic and progressive condition that involves demyelination of the myelinated neurones in the central nervous system. This is caused by an inflammatory process involving activation of immune cells against the myelin.

Answer 8

MS typically presents in young adults (under 50 years) and is more common women. Symptoms tend to improving in pregnancy and in the postpartum period.

Answer 9

Myelin covers the axons of neurones in the central nervous system. This myelin helps the electrical impulse move faster along the axon. Myelin is provided by cells that wrap themselves around the axons. These are Schwann cells in the peripheral nervous system and oligodendrocytes in the central nervous system. Multiple sclerosis typically only affects the central nervous system (the oligodendrocytes). There is inflammation around myelin and infiltration of immune cells that cause damage to the myelin. This affects the way electrical signals travel along the nerve leading to the symptoms of multiple sclerosis. When a patient presents with symptoms of a clinical “attack” of MS, for example an episode of optic neuritis, there are usually other lesions of demyelination at the same time throughout the central nervous system, most of which are not causing symptoms. In early disease, re-myelination can occur and symptoms can resolve. In the later stages of the disease, re-myelination is incomplete and symptoms gradually become more permanent. A characteristic features of MS is that lesions vary in their location over time, meaning that different nerves are affected and symptoms change over time. The key expression to remember to describe the way MS lesions change location over time is that they are “disseminated in time and space”.

Answer 10

The cause of the demyelination is unclear, but there is growing evidence that it is influenced by a combination of: - Multiple genes - Epstein–Barr virus (EBV) - Low vitamin D - Smoking - Obesity

Answer 11

Symptoms usually progress over more than 24 hours. At the first presentation symptoms tend to last days to weeks and then improve. There are a number of ways MS can present. These are described below. -Optic neuritis Eye movement abnormalities: - May present with diplopia (due to lesions affecting CN VI) - Internuclear opthalmoplegia - Conjugate lateral gaze disorder Focal weakness: - Bells palsy - Horners syndrome - Limb paralysis - Incontinence Focal sensory symptoms -Trigeminal neuralgia -Numbness -Paraesthesia (pins and needles) -Lhermitte’s sign is an electric shock sensation travels down the spine and into the limbs when flexing the neck. It indicates disease in the cervical spinal cord in the dorsal column. It is caused by stretching the demyelinated dorsal column. Ataxia: Ataxia is a problem with coordinated movement. It can be sensory or cerebellar: - Sensory ataxia is the result of loss of the proprioceptive sense, which is the ability to sense the position of the joint (e.g. is the joint flexed or extended). This results in a positive Romberg’s test and can cause pseudoathetosis. - Cerebellar ataxia is the result of problems with the cerebellum coordinating movement. This suggestions cerebellar lesions.

Answer 12

The disease course is highly variable between individuals. Some patients may have mild relapsing-remitting episodes for life whereas others have primary progressive MS that progresses without any improvement in symptoms. There are certain classifications used to describe the pattern of MS in an individual. These patterns are not separate conditions but part of a spectrum of disease activity. Clinically Isolated Syndrome This describes the first episode of demyelination and neurological signs and symptoms. MS cannot be diagnosed on one episode as the lesions have not been “disseminated in time and space”. Patients with clinically isolated syndrome may never have another episode or develop MS. If lesions are seen on MRI scan then they are more likely to progress to MS. Relapsing-Remitting Relapsing-remitting MS is the most common pattern at initial diagnosis. It is characterised by episodes of disease and neurological symptoms followed by recovery. In MS the symptoms occur in different areas with different episodes. This can be further classified based on whether the disease is active and/or worsening: Active: new symptoms are developing or new lesions are appearing on MRI Not active: no new symptoms or MRI lesions are developing Worsening: there is an overall worsening of disability over time Not worsening: there is no worsening of disability over time Secondary Progressive Secondary progressive MS is where there was relapsing-remitting disease at first, but now there is a progressive worsening of symptoms with incomplete remissions. Symptoms become more and more permanent. Secondary progressive MS can be further classified based on whether the disease is active and/or progressing. Active: new symptoms are developing or new lesions are appearing on MRI Not active: no new symptoms or MRI lesions are developing Progressing: there is an overall worsening of disease over time (regardless of relapses) Not progressing: there is no worsening of disease over time Primary Progressive Primary progressive MS is where there is a worsening of disease and neurological symptoms from the point of diagnosis without initial relapses and remissions. This can be further classified in a similar way to secondary progressive based on whether it is active and/or progressing.

Answer 13

Diagnosis is made by a neurologist based on the clinical picture and symptoms suggesting lesions that change location over time. Symptoms have to be progressive over a period of 1 year to diagnose primary progressive MS. Other causes for the symptoms need to be excluded. Investigations can support the diagnosis: - MRI scans can demonstrate typical lesions - Lumbar puncture can detect “oligoclonal bands” in the cerebrospinal fluid (CSF)

Answer 14

Optic neuritis presents with unilateral reduced vision developing over hours to days. Key features are: - Central scotoma. This is an enlarged blind spot. - Pain on eye movement - Impaired colour vision - Relative afferent pupillary defect

Answer 15

- Sarcoidosis - Systemic lupus erythematosus - Diabetes - Syphilis - Measles - Mumps - Lyme disease

Answer 16

Patients presenting with acute loss of vision should be seen urgently by an ophthalmologist for assessment. It is treated with steroids and recovery takes 2-6 weeks. Around 50% of patients with a single episode of optic neuritis will go on to develop MS over the next 15 years. Changes on an MRI scan help to predict which patients will go on to develop MS.

Answer 17

Multiple sclerosis is managed by a specialist multidisciplinary team (MDT) including neurologists, specialist nurses, physiotherapy, occupational therapy and others. Patient should be fully educated about their condition and treatment. Disease Modification Treatment with disease-modifying drugs and biologic therapy has changed the management of multiple sclerosis so that the aim of treatment is now to induce long term remission with no evidence of disease activity. There are many options that target various mechanisms such as interleukins, inflammatory cytokines and various immune cells. Going into detail about these drugs is beyond what would be expected in your exams. Disease-modifying drug treatment will be coordinated by specialists in multiple sclerosis. Treating Relapses: Relapses can be treated with steroids. NICE recommend methylprednisolone: -500mg orally daily for 5 days -1g intravenously daily for 3–5 days where oral treatment has failed previously or where relapses are severe Symptomatic Treatments It is important to treat the symptoms that result from the disease process along with treating the disease process itself: - Exercise to maintain activity and strength - Neuropathic pain can be managed with medication such as amitriptyline or gabapentin - Depression can be managed with antidepressants such as SSRIs - Urge incontinence can be managed with anticholinergic medications such as tolterodine or oxybutynin (although be aware these can cause or worsen cognitive impairment) - Spasticity can be managed with baclofen, gabapentin and physiotherapy

Answer 18

An umbrella term that encompasses a variety of specific diagnoses. Note: Motor neurone disease is a progressive, ultimately fatal condition where the motor neurones stop functioning. There is no effect on the sensory neurones and patients should not experience any sensory symptoms.

Answer 19

- Amylotropic lateral sclerosis (AML) is the most common and well known specific type of motor neurone disease. Stephen Hawking had amylotropic lateral sclerosis. - Progressive bulbar palsy is the second most common form of motor neurone disease. It affects primarily the muscles of talking and swallowing. - Other types of motor neurone disease to be aware of are progressive muscular atrophy and primary lateral sclerosis.

Answer 20

There is progressive degeneration of both upper and lower motor neurones. The sensory neurones are spared.

Answer 21

The typical patient is a late middle aged (e.g. 60) man, possibly with an affected relative. There is an insidious, progressive weakness of the muscles throughout the body affecting the limbs, trunk, face and speech. The weakness is often first noticed in the upper limbs. There may be increased fatigue when exercising. They may complain of clumsiness, dropping things more often or tripping over. They can develop slurred speech (dysarthria).

Answer 22

Signs of lower motor neurone disease: - Muscle wasting - Reduced tone - Fasciculations (twitches in the muscles) - Reduced reflexes Signs of upper motor neurone disease: - Increased tone or spasticity - Brisk reflexes - Upgoing plantar responses

Answer 23

The diagnosis of motor neurone disease needs to be made very carefully. It is based on the clinical presentation and excluding other conditions that can cause motor neurone symptoms. It should only be made by a specialist when there is certainty. Unfortunately, the diagnosis is often delayed, which causes considerable anxiety and stress.

Answer 24

Unfortunately, there are no effective treatments for halting or reversing the progression of the disease. Riluzole can slow the progression of the disease and extend survival by a few months in AML. It is licensed in the UK and should be initiated by a specialist. Edaravone is currently used in the United States but not the UK. Recent studies suggest it has the potential to slow the progression of the disease and it may come in to use in the future. Non-invasive ventilation (NIV) used at home to support breathing at night improves survival and quality of life. The key to management of the condition is supporting the person and their family: - Effectively breaking bad news - Involving the multidisciplinary team (MDT) in supporting and maintaining their quality of life - Advanced directives to document the patients wishes as the disease progresses - End of life care planning - Patients usually die of respiratory failure or pneumonia

Answer 25

Parkinson’s disease is a condition where there is a progressive reduction of dopamine in the basal ganglia of the brain, leading to disorders of movement.

Answer 26

- Resting tremor - Rigidity - Bradykinesia The symptoms are characteristically asymmetrical, with one side affected more than the other.

Answer 27

The basal ganglia are a group of structures situated in the middle of the brain. They is responsible for coordinating habitual movements such as walking or looking around, controlling voluntary movements and learning specific movement patterns. Part of the basal ganglia called the substantia nigra produces a neurotransmitter called dopamine. Dopamine is essential for the correct functioning of the basal ganglia. In Parkinson’s disease, there is a gradual but progressive fall in the production of dopamine due to the destruction of the substantia nigra.

Answer 28

The typical patient is an older aged man around the age of 70. Unilateral Tremor: The tremor in Parkinsons has a frequency of 4-6 Hz, meaning it occurs 4-6 times a second. This is described as a “pill rolling tremor” because it looks like they are rolling a pill between their fingertips and thumb. It is more pronounced when resting and improves on voluntary movement. The tremor is worsened if the patient is distracted. Asking them to do a task with the other hand, such as miming the motion of painting a fence, can exaggerate the tremor. “Cogwheel” Rigidity: Rigidity is a resistance to passive movement of a joint. If you take their hand and passively flex and extend their arm at the elbow, you will feel a tension in their arm that gives way to movement in small increments (like little jerks). This is what leads to the cogwheel description. Bradykinesia Bradykinesia describes how their movements get slower and smaller. This presents in a number of ways: - Their handwriting gets smaller and smaller (this is a classic presenting complaint in exams) - They can only take small steps when walking (“shuffling gait”) - They have difficulty initiating movement (e.g. from standing still to walking) - They have difficulty in turning around when standing, having to take lots of little steps - They have reduced facial movements and facial expressions (hypomimia) Other Features: There are a number of other features that often affect patients with Parkinson’s disease: - Depression - Sleep disturbance and insomnia - Loss of the sense of smell (anosmia) - Postural instability - Cognitive impairment and memory problems

Answer 29

A common exam task challenges you to distinguish between the tremor of Parkinson’s disease and benign essential tremor. The table below gives some tips on this: Parkinson’s Tremor: - Asymmetrical - 4-6 hertz - Worse at rest - Improves with intentional movement - Other Parkinson’s features - No change with alcohol Benign essential tremor: - Symmetrical - 5-8 hertz - Improves at rest - Worst with intentional movement - No other Parkinson's features - Improves with alcohol

Answer 30

Multiple System Atrophy This is a rare condition where the neurones of multiple systems in the brain degenerate. It affects the basal ganglia as well as multiple other areas. The degeneration of the basal ganglia lead to a Parkinson’s presentation. The degeneration in other areas lead to autonomic dysfunction (causing postural hypotension, constipation, abnormal sweating and sexual dysfunction) and cerebellar dysfunction (causing ataxia). Dementia with Lewy Bodies This is a type of dementia associated with features of Parkinsonism. It causes a progressive cognitive decline. There are associated symptoms of visual hallucinations, delusions, disorders of REM sleep and fluctuating consciousness. Others Two other Parkinson’s-plus syndromes exist that involves a number of complex progressive neurological features: Progressive Supranuclear Palsy Corticobasal Degeneration

Answer 31

Parkinson’s disease is diagnosed clinically based on symptoms and examination. The diagnosis should be made by a specialist with experience in diagnosing Parkinson’s. NICE recommend using the UK Parkinson’s Disease Society Brain Bank Clinical Diagnostic Criteria.

Answer 32

Treatment is initiated and guided by a specialist, and is tailored to each individual patient and their response to different medications. There is no cure, so treatment is focused on controlling symptoms and minimising side effects. Patients describe themselves as “on” when the medications are acting and they are moving freely, and “off” when the medications wear out, they have significant symptoms and their next dose is due.

Answer 33

Levodopa This is synthetic dopamine given orally to boost their own dopamine levels. It is usually combined with a drug that stops levodopa being broken down in the body before it gets the chance to enter the brain. These are peripheral decarboxylase inhibitors. Examples are carbidopa and benserazide. Combination drugs are: Co-benyldopa (levodopa and benserazide) Co-careldopa (levodopa and carbidopa) Levodopa is the most effective treatment for symptoms but becomes less effective over time. It is often reserved for when other treatments are not managing to control symptoms. The main side effect of dopamine is when the dose is too high patients develop dyskinesias. Theses are abnormal movements associated with excessive motor activity. Examples are: Dystonia: This is where excessive muscle contraction leads to abnormal postures or exaggerated movements. Chorea: These are abnormal involuntary movements that can be jerking and random. Athetosis: These are involuntary twisting or writhing movements usually in the fingers, hands or feet. COMT Inhibitors The main example of this is entacapone. These are inhibitors of catechol-o-methyltransferase (COMT). The COMT enzyme metabolises levodopa in both the body and brain. Entacapone is taken with levodopa (and a decarboxylase inhibitor) to slow breakdown of the levodopa in the brain. It extends the effective duration of the levodopa. Dopamine Agonists These mimic dopamine in the basal ganglia and stimulate the dopamine receptors. They are less effective than levodopa in reducing symptoms. They are usually used to delay the use of levodopa and are then used in combination with levodopa to reduce the dose of levodopa that is required to control symptoms. One notable side effect with prolonged use is pulmonary fibrosis. Examples are: Bromocryptine Pergolide Carbergoline Monoamine Oxidase-B Inhibitors Monoamine oxidase enzymes break down neurotransmitters such as dopamine, serotonin and adrenaline. The monoamine oxidase-B enzyme is more specific to dopamine and does not act on serotonin or adrenalin. These medications block this enzyme and therefore help increase the circulating dopamine. Similarly to dopamine agonists, they are usually used to delay the use of levodopa and then in combination with levodopa to reduce the required dose. Examples are: Selegiline Rasagiline

Answer 34

It is characterised by a fine tremor affecting all the voluntary muscles. It is most notable in the hands but affects other areas, for example causing a head tremor, jaw tremor and vocal tremor.

Answer 35

- Fine tremor - Symmetrical - More prominent on voluntary movement - Worse when tired, stressed or after caffeine - Improved by alcohol - Absent during sleep

Answer 36

- Parkinson’s disease - Multiple sclerosis - Huntington’s Chorea - Hyperthyroidism - Fever - Medications (e.g. antipsychotics)

Answer 37

There is no definitive treatment for benign essential tremor. The tremor is not harmful and does not require treatment if not causing functional or psychological problems. Medications that can be tried to improve symptoms are: - Propranolol (a non-selective beta blocker) - Primidone (a barbiturate anti-epileptic medication)

Answer 38

Epilepsy is an umbrella term for a condition where there is a tendency to have seizures. Seizures are transient episodes of abnormal electrical activity in the brain. There are many different types of seizures. A diagnosis of epilepsy is made by a specialist based on the characteristics of the seizure episodes.

Answer 39

- Electroencephalogram (EEG) - MRI brain - can be used to visualise the structure of the brain. It is used to diagnose structural problems that may be associated with seizures and other pathology such as tumours Other investigations can be used to exclude other pathology, particularly and ECG to exclude problems in the heart.

Answer 40

- Generalised Tonic-Clonic Seizures - Focal Seizures - Absence Seizures - Atonic Seizures - Myoclonic Seizures - Infantile spasms

Answer 41

These are what most people think of with an epileptic seizure. There is loss of consciousness and tonic (muscle tensing) and clonic (muscle jerking) episodes. Typically the tonic phase comes before the clonic phase. There may be associated tongue biting, incontinence, groaning and irregular breathing. After the seizure there is a prolonged post-ictal period where the person is confused, drowsy and feels irritable or depressed. Management of tonic-clonic seizures is with: - First line: sodium valproate - Second line: lamotrigine or carbamazepine

Answer 42

Focal seizures start in temporal lobes. They affect hearing, speech, memory and emotions. There are various ways that focal seizures can present: Hallucinations Memory flashbacks Déjà vu Doing strange things on autopilot One way to remember the treatment is that they are the reverse of tonic-clonic seizures: - First line: carbamazepine or lamotrigine - Second line: sodium valproate or levetiracetam

Answer 43

Absence seizures typically happen in children. The patient becomes blank, stares into space and then abruptly returns to normal. During the episode they are unaware of their surroundings and won’t respond. These typically only lasts 10-20 seconds. Most patients (> 90%) stop having absence seizures as they get older. Management is: -First line: sodium valproate or ethosuximide

Answer 44

Atonic seizures are also known as “drop attacks”. They are characterised by brief lapses in muscle tone. These don’t usually last more than 3 minutes. They typically begin in childhood. They may be indicative of Lennox-Gastaut syndrome. Management is: - First line: sodium valproate - Second line: lamotrigine

Answer 45

Myoclonic seizures present as sudden brief muscle contractions, like a sudden “jump”. The patient usually remains awake during the episode. They occur in various forms of epilepsy but typically happen in children as part of juvenile myoclonic epilepsy. Management is: - First line: sodium valproate - Other options: lamotrigine, levetiracetam or topiramate

Answer 46

This is also known as West syndrome. It is a rare (1 in 4000) disorder starting in infancy at around 6 months of age. It is characterised by clusters of full body spasms. There is a poor prognosis: 1/3 die by age 25, however 1/3 are seizure free. It can be difficult to treat but first line treatments are: - Prednisolone - Vigabatrin

Answer 47

- Sodium valproate - Carbamazepine - Phenytoin - Ethosuximide - Lamotrigine

Answer 48

This is a first line option for most forms of epilepsy (except focal seizures). It works by increasing the activity of GABA, which has a relaxing effect on the brain. Notable side effects: - Teratogenic so patients need careful advice about contraception - Liver damage and hepatitis - Hair loss - Tremor Note: There are a lot of warning about the teratogenic effects of sodium valproate and NICE updated their guidelines in 2018 to reflect this. It must be avoided in girls or women unless there are no suitable alternatives and strict criteria are met to ensure they do not get pregnant.

Answer 49

This is first line for focal seizures. Notable side effects are: - Agranulocytosis - Aplastic anaemia - Induces the P450 system so there are many drug interactions

Answer 50

- Folate and vitamin D deficiency - Megaloblastic anaemia (folate deficiency) - Osteomalacia (vitamin D deficiency)

Answer 51

- Night terrors | - Rashes

Answer 52

- Stevens-Johnson syndrome or DRESS syndrome. These are life threatening skin rashes - Leukopenia

Answer 53

Seizures lasting more than 5 minutes or more than 3 seizures in one hour.

Answer 54

Management of status epileptics in the hospital: Take an ABCDE approach: - Secure the airway - Give high-concentration oxygen - Assess cardiac and respiratory function - Check blood glucose levels - Gain intravenous access (insert a cannula) - IV lorazepam 4mg, repeated after 10 minutes if the seizure continues - If seizures persist: IV phenobarbital or phenytoin Medical options in the community: - Buccal midazolam - Rectal diazepam

Answer 55

Abnormal functioning of the sensory nerves, delivering abnormal and painful signals to the brain.

Answer 56

Paresthesia

Answer 57

- Postherpetic neuralgia from shingles is in the distribution of a dermatome and usually on the trunk - Nerve damage from surgery - Multiple sclerosis - Diabetic neuralgia typically affects the feet - Trigeminal neuralgia - Complex Regional Pain Syndrome (CRPS)

Answer 58

- Paresthesia - Electric shocks - Loss of sensation to touch of the affected area

Answer 59

DN4 questionnaire. They are scored out of 10. A score of 4 or more indicates neuropathic pain.

Answer 60

There are four first line treatments for neuropathic pain: - Amitriptyline is a tricyclic antidepressant - Duloxetine is an SNRI antidepressant - Gabapentin is an anticonvulsant - Pregabalin is an anticonvulsant NICE recommend using one of these four medications to control neuropathic pain. If that does not work then stop and start an alternative and repeat this until all four have been tried. Other options: - Tramadol ONLY as a rescue for short term control of flares - Capsaicin cream (chilli pepper cream) for localised areas of pain - Physiotherapy to maintain strength - Psychological input to help with understanding and coping

Answer 61

A type of neuropathic pain. Carbamazepine is the first line recommended treatment. If that doesn't work you should refer to a specialist.

Answer 62

This is a condition where areas are affected by abnormal nerve functioning causing neuropathic pain and abnormal sensations. It is usually isolated to one limb. Often it is triggered by an injury to the area. The area can become very painful and hypersensitive even to simple inputs such as wearing clothing. It can also intermittently swell, change colour, change temperature, flush with blood and have abnormal sweating. Treatment is often guided by a pain specialist and is similar to other neuropathic pain.

Answer 63

At the cerebellopontine angle. It passes through the temporal bone and parotid gland.

Answer 64

- Temporal - Zygomatic - Buccal - Marginal mandibular - Cervical

Answer 65

There are three functions of the facial nerve: motor, sensory and parasympathetic. Motor: Supplies the muscles of facial expression, the stapedius in the inner ear and the posterior digastric, stylohyoid and platysma muscles in the neck. Sensory: carries taste from the anterior 2/3 of the tongue. Parasympathetic: it provides the parasympathetic supply to the submandibular and sublingual salivary glands and the lacrimal gland (stimulating tear production).

Answer 66

- Cerebrovascular accidents (strokes) | - Tumours

Answer 67

- Pseudobulbar palsies | - Motor neurone disease

Answer 68

It presents as a unilateral lower motor neurone facial nerve palsy. The majority of patients fully recover over several weeks but recovery may take up to 12 months. A third are left with some residual weakness.

Answer 69

- If patients present within 72 hours of developing symptoms, NICE guidelines recommend considering prednisolone as treatment, either: - 50mg for 10 days - 60mg for 5 days followed by a 5-day reducing regime of 10mg a day -Patients also require lubricating eye drops to prevent the eye on the affected drying out and being damaged. If they develop pain in the eye they need ophthalmology review for exposure keratopathy. Tape can be used to keep the eye closed at night.

Answer 70

It presents as a unilateral lower motor neurone facial nerve palsy. Patients stereotypically have a painful and tender vesicular rash in the ear canal, pinna and around the ear on the affected side. This rash can extend to the anterior 2/3 of the tongue and hard palate.

Answer 71

The herpes zoster virus

Answer 72

Treatment should ideally be initiated within 72 hours. Treatment is with: - Prednisolone - Aciclovir Patients also require lubricating eye drops.

Answer 73

Ramsay-Hunt syndrome is a very popular presentation in your MCQ exams. Look out for that patient with a vesicular rash around their ear and a facial nerve palsy.

Answer 74

Infection: - Otitis media - Malignant otitis externa - HIV - Lyme’s disease Systemic disease: - Diabetes - Sarcoidosis - Leukaemia - Multiple sclerosis - Guillain–Barré syndrome Tumours: - Acoustic neuroma - Parotid tumours - Cholesteatomas Trauma: - Direct nerve trauma - Damage during surgery - Base of skull fractures

Answer 75

Brain tumours are abnormal growths within the brain. There are many different types of brain tumour. They vary from benign tumours (e.g. meningiomas) to highly malignant (e.g. glioblastomas). Often brain tumours do not have any symptoms, particularly when they are small. As they develop they present with focal neurological symptoms depending on the location of the lesion. Brain tumours often present with symptoms and signs of raised intracranial pressure. As a tumour grows within the skull it takes up space. This leaves less space for the other contents of the skull (such as the CSF) to squeeze in to and leads to a rise in the pressure within the intracranial space.

Answer 76

A common exam question asks the location of the lesion based on the neurology. A popular exam question describes a patient that has had an unusual change in personality and behaviour. This indicates a tumour in the frontal lobe. Remember that the frontal lobe is responsible for personality and higher-level decision making.

Answer 77

Papilloedema

Answer 78

- Brain tumours - Intracranial haemorrhage - Idiopathic intracranial hypertension - Abscesses or infection

Answer 79

Concerning features of a headache that should prompt further examination and investigation include: - Constant - Nocturnal - Worse on waking - Worse on coughing, straining or bending forward - Vomiting Other presenting features of raised intracranial pressure may be: - Altered mental state - Visual field defects - Seizures (particularly focal) - Unilateral ptosis - Third and sixth nerve palsies - Papilloedema (on fundoscopy)

Answer 80

Swelling of the optic disc secondary to raised ICP

Answer 81

The sheath around the optic nerve is connected with the subarachnoid space. Therefore it is possible for CSF under high pressure to flow into the optic nerve sheath. This increases the pressure around the optic nerve where it connects with the back of the eye at the optic disc, causing optic disc swelling.

Answer 82

- Secondary metastases - Gliomas - Meningiomas - Pituitary tumours - Acoustic neuroma (AKA vestibular schwannoma)

Answer 83

- Lung - Breast - Colorectal - Prostate

Answer 84

Tumours of glial cells in the brain or spinal cord. They are graded from 1 to 4 (grade 4 are the most malignant). Three types to remember: - Astrocytoma (glioblastoma multiforme is the most common) - Oligodendroglioma - Ependymoma

Answer 85

Tumours growing from the cells of the meninges in the brain and spinal cord. They are usually benign, however they take up space and this mass effect can lead to raised intracranial pressure and neurological symptoms.

Answer 86

Usually benign

Answer 87

They can compress on the optic chiasm causing a specific visual field defect called a bitemporal hemianopia.

Answer 88

They have the potential to cause hormone deficiencies (hypopituitarism) or to release excessive hormones leading to: - Acromegaly - Hyperprolactinaemia - Cushing’s disease - Thyrotoxicosis

Answer 89

Tumours of the schwann cells surrounding the auditory nerve that innervates the inner ear.

Answer 90

Around the cerebellopontine angle.

Answer 91

Usually unilateral. Note: Bilateral acoustic neuromas are associated with neurofibromatosis type 2

Answer 92

- Hearing loss - Tinnitus - Balance problems They can be associated with a facial nerve palsy

Answer 93

There is massive variation in brain tumours from completely benign to extremely malignant. Surgery is dependent on the grade and behaviour of the brain tumour. Management options include: - Palliative care - Chemotherapy - Radiotherapy - Surgery

Answer 94

- Trans-sphenoidal surgery - Radiotherapy - Bromociptine to block prolactin secreting tumours - Somatostatin analogues (e.g. ocreotide) to block growth hormone secreting tumours

Answer 95

An autosomal dominant genetic condition that causes a progressive deterioration in the nervous system. Patients are usually asymptomatic until symptoms begin around aged 30 to 50.

Answer 96

“trinucleotide repeat disorder” HTT gene 4

Answer 97

- An earlier age of onset | - Increased severity of disease

Answer 98

Anticipation is a common topic of exam questions. It is worth remembering the features and connection with Huntington’s for your exams.

Answer 99

It usually presents with an insidious, progressive worsening of symptoms. It typically begins with cognitive, psychiatric or mood problems. These are followed by the development of movement disorders; - Chorea (involuntary, abnormal movements) - Eye movement disorders - Dysarthria - Dysphagia

Answer 100

Diagnosis is made in a specialist genetic centre using a genetic test for the faulty gene. It involves pre-test and post-test counselling regarding the implications of the results.

Answer 101

There are currently no treatment options for slowing or stopping the progression of the disease. The key to management of the condition is supporting the person and their family: -Effectively breaking bad news -Involvement of MDT in supporting and maintaining their quality of life (e.g. occupational therapy, physiotherapy and psychology) -Speech and language therapy where there are speech and swallowing difficulties -Genetic counselling regarding relatives, pregnancy and children -Advanced directives to document the patients wishes as the disease progresses End of life care planning Medical treatment is based on symptomatic relief. As the disease progresses medication requirements may change. It is important to discontinue unnecessary medication to minimise adverse effects. Medications that can suppress the disordered movement: - Antipsychotics (e.g. olanzapine) - Benzodiazepines (e.g. diazepam) - Dopamine-depleting agents (e.g. tetrabenazine) Depression can be treated with antidepressants.

Answer 102

Huntington’s chorea is a progressive condition. Life expectance is around 15-20 years after the onset of symptoms. As the disease progresses patients become more susceptible and less able to fight off illnesses. Death is often due to respiratory disease (e.g. pneumonia). Suicide is a more common cause of death than in the general population.

Answer 103

Myasthenia graves is an autoimmune condition that causes muscle weakness that gets progressively worse with activity and improves with rest.

Answer 104

Myasthenia gravis affects men and women at different ages. Typical patients are either a woman under the age of 40 or a man over the age of 60.

Answer 105

In around 85% of patients with myasthenia gravis, acetylcholine receptor antibodies are produced by the immune system. These bind to the postsynaptic neuromuscular junction receptors. This blocks the receptor and prevents the acetylcholine from being able to stimulate the receptor and trigger muscle contraction. As the receptors are used more during muscle activity, more of them become blocked up. This leads to less effective stimulation of the muscle with increased activity. There is more muscle weakness the more the muscles are used. This improves with rest as more receptors are freed up for use again. These antibodies also activate the complement system within the neuromuscular junction, leading to damage to cells at the postsynaptic membrane. This further worsens the symptoms. There are two other antibodies that cause the other 15% of cases of myasthenia gravis. These are antibodies against muscle-specific kinase (MuSK) and antibodies against low-density lipoprotein receptor-related protein 4 (LRP4). MuSK and LRP4 and important proteins for the creation and organisation of the acetylcholine receptor. Destruction of these proteins by autoantibodies leads to inadequate acetylcholine receptors. This causes the symptoms of myasthenia gravis.

Answer 106

The severity of symptoms can vary dramatically between patients. They can be mild and subtle or life threateningly severe. The characteristic feature is weakness that gets worse with muscle use and improves with rest. Symptoms are typically minimal in the morning and worst at the end of the day. The symptoms most affect the proximal muscles and small muscles of the head and neck. It leads to: - Extraocular muscle weakness causing diplopia - Eyelid weakness causing ptosis - Weakness in facial movements - Dysphagia - Fatigue in the jaw when chewing - Dysarthria - Progressive weakness with repetitive movements

Answer 107

There are a few ways to elicit fatiguability in the muscles: - Repeated blinking will exacerbate ptosis - Prolonged upward gazing will exacerbate diplopia on further eye movement testing - Repeated abduction of one arm 20 times will result in unilateral weakness when comparing both sides Check for a thymectomy scar. Test the forced vital capacity (FVC).

Answer 108

Diagnosis can be made testing directly for the relevant antibodies: - Acetylcholine receptor (ACh-R) antibodies (85% of patients) - Muscle-specific kinase (MuSK) antibodies (10% of patients) - LRP4 (low-density lipoprotein receptor-related protein 4) antibodies (less than 5%) A CT or MRI of the thymus gland is used to look for a thymoma. The edrophonium test can be helpful where there is doubt about the diagnosis

Answer 109

Patients are given an IV dose of edrophonium chloride (or neostigmine). Normally, cholinesterase enzymes in the neuromuscular junction break down acetylcholine. Edrophonium block these enzymes and stop the breakdown of acetylcholine. As a result the level of acetylcholine at the neuromuscular junction increases. It briefly and temporarily relieves the weakness. This establishes a diagnosis of myasthenia gravis.

Answer 110

- Reversible acetylcholinesterase inhibitors (usually pyridostigmine or neostigmine) increases the amount of acetylcholine in the neuromuscular junction and improve symptoms - Immunosuppression (e.g. prednisolone or azathioprine) suppresses the production of antibodies - Thymectomy can improve symptoms even in patients without a thymoma Monoclonal antibodies: - Rituximab is a monoclonal antibody that targets B cells and reduces the production of antibodies. It is available on the NHS if standard treatment is not effective and certain criteria are met. - Eculizumab is a monoclonal antibody that targets complement protein C5. This could potentially prevent the complement activation and destruction of acetylcholine receptors. There is ongoing research and debate about whether the evidence is strong enough to offer it on the NHS. It is currently not recommended by NICE.

Answer 111

A severe complication of myasthenia gravis. It can be life threatening. It causes an acute worsening of symptoms, often triggered by another illness such as a respiratory tract infection. This can lead to respiratory failure as a result of weakness in the muscle of respiration

Answer 112

Patients may require non-invasive ventilation with BiPAP or full intubation and ventilation. Medical treatment is with immunomodulatory therapies such as IV immunoglobulins and plasma exchange.

Answer 113

Myasthenia gravis

Answer 114

Progressive muscle weakness with increased use

Answer 115

The symptoms tend to be more insidious and less pronounced than in myasthenia gravis.

Answer 116

Small-cell lung cancer

Answer 117

It is a result of antibodies produced by the immune system against voltage-gated calcium channels in small cell lung cancer (SCLC) cells. These antibodies also target and damage voltage-gated calcium channels in the presynaptic terminals of the neuromuscular junction where motor nerves communicate with muscle cells. These voltage-gated calcium channels are responsible for assisting in the release of acetylcholine into the synapse of the neuromuscular junction. This acetylcholine then binds to the acetylcholine receptors and stimulates a muscle contraction. When these channels are destroyed, less acetylcholine is release into the synapse.

Answer 118

Symptoms tend to develop slowly. The proximal muscles are most notably affected causing proximal muscle weakness. It can also affect - The intraocular muscles causing DIPLOPIA - The levator muscles in the eyelid causing PTOSIS - The oropharyngela muscles causing slurred speech and DYSPHAGIA This weakness is worse with prolonged use of the muscles

Answer 119

It is important to diagnose and manage any underlying malignancy. In older smokers with symptoms of Lambert-Eaton syndrome consider investigating for small cell lung cancer. Amifampridine allows more acetylcholine to be released in the neuromuscular junction synapses. It works by blocking voltage-gated potassium channels in the presynaptic cells, which in turn prolongs the depolarisation of the cell membrane and assists calcium channels in carrying out their action. This improves symptoms in Lambert-Eaton syndrome. Other options: - Immunosuppressants (e.g. prednisolone or azathioprine) - IV immunoglobulins - Plasmapheresis

Answer 120

An inherited disease that affects the peripheral motor and sensory nerves.

Answer 121

There are various types of Charcot-Marie-Tooth with different genetic mutations and different pathophysiology. They cause dysfunction in the myelin or the axons. The majority of mutations are inherited in an autosomal dominant pattern. Symptoms usually start to appear before the age of 10 years but the onset of symptoms can be delayed until 40 or later.

Answer 122

- High foot arches (pes cavus) - Distal muscle wasting causing “inverted champagne bottle legs” - Weakness in the lower legs, particularly loss of ankle dorsiflexion - Weakness in the hands - Reduced tendon reflexes - Reduced muscle tone - Peripheral sensory loss

Answer 123

``` A – Alcohol B – B12 deficiency C – Cancer and Chronic Kidney Disease D – Diabetes and Drugs (e.g. isoniazid, amiodarone and cisplatin) E – Every vasculitis ```

Answer 124

A patient presenting with Charcot-Marie-Tooth disease is a common OSCE scenario. You will have to perform a neurological examination on a patient that has a peripheral neuropathy. Charcot-Marie-Tooth is a relatively common (1 in 2,500 people) condition with good signs that has a high chance of appearing in your exams. Look for the other features, suggest the diagnosis, then run through the ABCDE mnemonic to suggest the possible other causes.

Answer 125

There is no treatment to alter the underlying disease or prevent it progressing. Management is purely supportive with input from various members of the multidisciplinary team: - Neurologists and geneticists to make the diagnosis - Physiotherapists to maintain muscle strength and joint range of motion - Occupational therapists to assist with activities of living - Podiatrists to help with foot symptoms and suggest insoles and other orthoses to improve symptoms - Orthopaedic surgeons to correct disabling joint deformities

Answer 126

It is an “acute paralytic polyneuropathy” that affects the peripheral nervous system. It causes acute, symmetrical, ascending weakness and can also cause sensory symptoms. It is usually triggered by an infection and is particularly associated with to campylobacter jejuni, cytomegalovirus and Epstein-Barr virus.

Answer 127

Guillain-Barré is thought to occur due to a process called molecular mimicry. The B cells of the immune system create antibodies against the antigens on the pathogen that causes the preceding infection. These antibodies also match proteins on the nerve cells. They may target proteins on the myelin sheath of the motor nerve cell or the nerve axon.

Answer 128

- Symmetrical ascending weakness (starting at the feet and moving up the body) - Reduced reflexes - There may be peripheral loss of sensation or neuropathic pain - It may progress to the cranial nerves and cause facial nerve weakness

Answer 129

Symptoms usually start within 4 weeks of the preceding infection. The symptoms typically start in the feet and progresses upward. Symptoms peak within 2-4 weeks, then there is a recovery period that can last months to years.

Answer 130

A diagnosis of Guillain-Barré syndrome is made clinically. The Brighton criteria can be used for diagnosis. Diagnosis can be supported by investigations: - Nerve conduction studies (reduced signal through the nerves) - Lumbar puncture for CSF (raised protein with a normal cell count and glucose)

Answer 131

- IV immunoglobulins - Plasma exchange (alternative to IV IG) - Supportive care - VTE prophylaxis (pulmonary embolism is a leading cause of death) In severe cases with respiratory failure patients may need intubation, ventilation and admission to the intensive care unit.

Answer 132

- 80% will fully recover - 15% will be left with some neurological disability - 5% will die

Answer 133

A genetic condition that causes nerve tumours (neuromas) to develop throughout the nervous system. These tumours are benign, however they do cause neurological and structural problems

Answer 134

Type 1 and type 2.

Answer 135

The NF1 gene is found on chromosome 17. It codes for a protein called neurofibromin which is a tumour suppressor protein. Inheritance of this gene is AD.

Answer 136

There must be at least 2 of the 7 features to indicate a diagnosis. You can remember this with the mnemonic CRABBING. C – Café-au-lait spots (6 or more) measuring ≥ 5mm in children or ≥ 15mm in adults R – Relative with NF1 A – Axillary or inguinal freckles BB – Bony dysplasia such as Bowing of a long bone or sphenoid wing dysplasia I – Iris hamartomas (Lisch nodules) (2 or more) are yellow brown spots on the iris N – Neurofibromas (2 or more) or 1 plexiform neurofibroma G – Glioma of the optic nerve

Answer 137

Diagnosis is based on clinical criteria and no investigations are required to make a definitive diagnosis. Genetic testing can be used where there is doubt. Xrays can be used to investigate bone pain and bone lesions. Imaging with CT and MRI scans can be used to investigate lesions in the brain, spinal cord and elsewhere in the body.

Answer 138

There is no treatment of the underlying disease process or to prevent the development of neurofibromas or complications. Management is to control symptoms, monitor the disease and treat complications.

Answer 139

- Migraines - Epilepsy - Renal artery stenosis causing hypertension - Learning and behavioural problems (e.g. ADHD) - Scoliosis of the spine - Vision loss (secondary to optic nerve gliomas) - Malignant peripheral nerve sheath tumours - Gastrointestinal stromal tumour (a type of sarcoma) - Brain tumours - Spinal cord tumours with associated neurology (e.g. paraplegia) - Increased risk of cancer (e.g. breast cancer) - Leukaemia

Answer 140

The neurofibromatosis type 2 gene is found on chromosome 22. It codes for a protein called merlin, which is a tumour suppressor protein particularly important in Schwann cells. Mutations in this gene lead to the development of schwannomas (benign nerve sheath tumours of the Schwann cells). Inheritance is autosomal dominant.

Answer 141

Neurofibromatosis type 2 is most associated with acoustic neuromas. These are tumours of the auditory nerve innervating the inner ear. Symptoms of an acoustic neuroma are: - Hearing loss - Tinnitus - Balance problems Note: Schwannomas can also develop in the brain and spinal cord with symptoms based on the location of the lesion. Surgery can be used to resect tumours although there is a risk or permanent nerve damage.

Answer 142

Bilateral acoustic neuromas almost certainly indicate neurofibromatosis type 2. This is a popular association in exams so worth remembering.

Answer 143

The development of hamartomas

Answer 144

Benign neoplastic growths of the tissue that they originate from

Answer 145

- Skin - Brain - Lungs - Heart - Kidneys - Eyes

Answer 146

- TSC1 gene on chromosome 9, which codes for hamartin | - TSC2 gene on chromosome 16, which codes for tuberin

Answer 147

Interact with each other to control the size and growth of cells. Abnormalities in one of these proteins leads to abnormal cell size and growth.

Answer 148

- Ash leaf spots are depigmented areas of skin shaped like an ash leaf - Shagreen patches are thickened, dimpled, pigmented patches of skin - Angiofibromas are small skin coloured or pigmented papules that occur over the nose and cheeks - Subungual fibromata are fibromas growing from the nail bed. They are usually circular painless lumps that grow slowly and displace the nail - Cafe-au-lait spots are light brown “coffee and milk” coloured flat pigmented lesions on the skin - Poliosis is an isolated patch of white hair on the head, eyebrows, eyelashes or beard

Answer 149

Neurological features: - Epilepsy - Learning disability and developmental delay Other features: - Rhabdomyomas in the heart - Gliomas (tumours of the brain and spinal cord) - Polycystic kidneys - Lymphangioleiomyomatosis (abnormal growth in smooth muscle cells, often affecting the lungs. These can cause cough, SOB, chest pain, haemoptysis and pneumothorax) - Retinal hamartomas

Answer 150

The classical presentation is a child presenting with epilepsy found to have skin features of tuberous sclerosis. It can also present in adulthood.

Answer 151

Management is supportive with monitoring and treating complications such as epilepsy. There is no treatment for the underlying gene defect.

Answer 152

- Tension headaches - Migraines - Cluster headaches - Secondary headaches - Sinusitis - Giant cell arteritis - Glaucoma - Intracranial haemorrhage - Subarachnoid haemorrhage - Analgesic headache - Hormonal headache - Cervical spondylosis - Trigeminal neuralgia - Raised intracranial pressure (brain tumours) - Meningitis - Encephalitis

Answer 153

- Fever, photophobia or neck stiffness (meningitis or encephalitis) - New neurological symptoms (haemorrhage, malignancy or stroke) - Dizziness (stroke) - Visual disturbance (temporal arteritis or glaucoma) - Sudden onset occipital headache (subarachnoid haemorrhage) - Worse on coughing or straining (raised intracranial pressure) - Postural, worse on standing, lying or bending over (raised intracranial pressure) - Severe enough to wake the patient from sleep - Vomiting (raised intracranial pressure or carbon monoxide poisoning) - History of trauma (intracranial haemorrhage) - Pregnancy (pre-eclampsia)

Answer 154

Practice asking red flag questions so you can demonstrate in an exam that you are thinking about serious causes. This will score extra points and also help you document well when you start seeing patients.

Answer 155

Classically produce a mild ache across the forehead and in a band-like pattern around the head. They come on and resolve gradually and don't produce visual changes

Answer 156

- Stress - Depression - Alcohol - Skipping meals - Dehydration

Answer 157

- Reassurance - Basic analgesia - Relaxation techniques - Hot towels to local area

Answer 158

Secondary headaches give a similar presentation to a tension headache but with a clear cause.

Answer 159

- Underlying medical conditions such as infection, obstructive sleep apnoea or pre-eclampsia - Alcohol - Head injury - Carbon monoxide poisoning

Answer 160

Hormonal headaches are related to oestrogen. The produce a generic, non-specific, tension-like headache. They tend to be related to low oestrogen: - Two days before and first three days of the menstrual period - Around the menopause - Pregnancy. It is worse in the first few weeks and improves in the last 6 months. Headaches in the second half of pregnancy should prompt investigation for pre-eclampsia. The oral contraceptive pill can improve hormonal headaches.

Answer 161

Cervical spondylosis is a common condition caused by degenerative changes in the cervical spine. It causes neck pain, usually made worse by movement. However, if often presents with headache. It is important to exclude other causes of neck pain such as inflammation, malignancy and infection. It is also important to exclude spinal cord or nerve root lesions.

Answer 162

- Ophthalmic (V1) - Maxillary (V2) - Mandibular (V3)

Answer 163

Trigeminal neuralgia can affect any combination of the branches. The cause is unclear but it is thought to be caused by compression of the nerve. 90% of cases are unilateral, 10% are bilateral. Around 5-10% of people with multiple sclerosis have trigeminal neuralgia.

Answer 164

It presents with intense facial pain that comes on spontaneously and last anywhere between a few seconds to hours. It is often described as an electricity-like shooting pain. Attacks often worsen over time. Note: There are a number of possible triggers for the pain in patients with trigeminal neuralgia. These include things like cold weather, spicy food, caffeine and citrus fruits.

Answer 165

NICE recommend carbamazepine as first-line for trigeminal neuralgia. Surgery to decompress or intentionally damage the trigeminal nerve is an option.

Answer 166

- Migraine without aura - Migraine with aura - Silent migraine (migraine with aura but without a headache) - Hemiplegic migraine

Answer 167

- Moderate to severe intensity - Pounding or throbbing in nature - Usually unilateral but can be bilateral - Photophobia - Phonophobia - With or without aura - Nausea and vomiting

Answer 168

- Sparks in vision - Blurring vision - Lines across vision - Loss of different visual fields

Answer 169

``` Typical migraine symptoms Sudden or gradual onset Hemiplegia (unilateral weakness of the limbs) Ataxia Changes in consciousness ```

Answer 170

- Stress - Bright lights - Strong smells - Certain foods (e.g. chocolate, cheese, caffeine) - Dehydration - Menstruation - Abnormal sleep patterns - Trauma

Answer 171

Patients often develop their own patterns for helping to relieve their symptoms. Often patients will go to a dark quiet room and sleep. Options for medical management are: - Paracetamol - Triptans (e.g. sumatriptan 50mg as the migraine starts) - NSAIDs (e.g ibuprofen or naproxen) - Antiemetics if vomiting occurs (e.g. metoclopramide)

Answer 172

5HT receptor agonists

Answer 173

Keeping a headache diary can help identify triggers Certain medications can be used long term to reduce the frequency and severity of attacks: - Propranolol - Topiramate (this is teratogenic and can cause a cleft lip/palate so patients should not get pregnant) - Amitriptyline Acupuncture is an option recommended by NICE recommend for the treatment of migraines. It is reported to be as effective as prophylactic medications. Supplementation with vitamin B2 (riboflavin) may reduce frequency and severity. In migraine specifically triggered around menstruation then prophylaxis with NSAIDS (e.g. mefanamic acid) or triptans (frovatriptan or zolmitriptan) can be used around that time as a preventative measure. Migraines tend to get better over time and people often go in to remission from their symptoms.

Answer 174

Severe and unbearable unilateral headaches, usually around the eye

Answer 175

The headaches come in clusters of attacks and then disappear for a while. For example, a patient may suffer 3 – 4 attacks a day for weeks or months followed by a pain-free period lasting 1-2 years. Attacks last between 15 minutes and 3 hours.

Answer 176

A typical patient with cluster headaches in your exams is a 30 – 50 year-old male smoker. Attacks can be triggered by things like alcohol, strong smells and exercise.

Answer 177

Symptoms are typically all unilateral: - Red, swollen and watering eye - Miosis - Ptosis - Nasal discharge - Facial sweating

Answer 178

Acute management: - Triptans (e.g. sumatriptan 6mg injected subcutaneously) - High flow 100% oxygen for 15-20 minutes (can be given at home) Prophylaxis options: - Verapamil - Lithium - Prednisolone (a short course for 2-3 weeks to break the cycle during clusters)